Embolic stroke after total cavopulmonary connection for complex congenital heart disease - A case report

Thrombosis has been reported to occur variably from 1 day to more than 10 years after Fontan-type operation. We herein describe a case with heterotaxy syndrome with situs ambiguous, dextrocardia, single ventricle, complete atrioventricular canal defect, malposition of great arteries and bilateral superior vena cava who had received pulmonary artery banding and total cavopulmonary connection at the ages of 2 months and 4 years, respectively. However, he was lost to follow-up. Eleven years after the operation, the patient suffered from acute cerebral infarction of right middle cerebral artery territory, which was documented by brain computed tomography. Multiple venous thrombi were detected by suprasternal approach of two-dimensional transthoracic echocardiography and cardiac angiography during hospitalization. The patient finally expired in spite of aggressive treatment. We suggest that transthoracic and/or even transesophageal echocardiography should be performed as a routine surveillance for cardiac function evaluation as well as for thrombus detection in patients after cavopulmonary connection. In addition, long-term use of oral anticoagulant may be necessary, especially when patients have low cardiac output and/or high atrial pressure.